Sunday, March 09, 2008

It is easy to imagine that you have Fatal Familial Insomnia (FFI). It starts with a strange turn: one patient described a sudden shock from head to toe and then vertigo. Then night-time fever follows, with the pupils of the eyes contracting to pin-pricks, and the neck becoming stiff; but the worst symptom of all is that the patient doesn't sleep. I guess everyone knows the some of the debilitating effects of insomnia - the tearfulness, the anxiety and agitation, the loss of memory and depression - and in FFI this goes on for months. In the end the patient is reduced to lying, exhausted and twitching and perfectly conscious, until some side-effect - like drowning in the patient's own saliva due to a non-working swallowing mechanism - is fatal. An autopsy reveals that the adrenal gland, which produces adrenaline and cortisol, is burnt out, as if the person lying on the bed 'has been running away from lions' for months on end. The only other clue is the thalamus which is full of holes. FFI, like scrapie in sheep, BSE in cattle and CJD and Kuru in man, is a disease caused by prions.

THE FAMILY THAT COULDN'T SLEEP by is the aptly but strangely titled history of prion discovery by D.T.Max. It is cleverly structured, rather like a literary novel: the family history of this disease from the earliest recorded case - a doctor living in Cassanova's eighteenth century Venice - to the modern day where his descendants wait for the ominous signs that they too are afflicted, is recorded in chapters which are interspersed with the general history of the discovery of prion disease. So far I have read about scrapie (coincidentally first recorded at about the time that the doctor lived), kuru and CJD. The way the mystery is unfolding is fascinating and wonderfully described. At the moment I am on chapter 8, in the 1970s and have to stop for breakfast...

10 Comments:

I've already been over to the library site and out an order in for this; it sounds fascinating. Nothing frightens me more than the idea of sleep deprivation. I have gone through bad sleeping patches in my life and know how destructive they can be. Maybe this will help me keep my own occasional difficulties in proportion.

I like books like this (I enjoyed 'Counting Sheep' all about sleep) - but I find that reading them makes me a bit anxious too. Its the old thing of thinking too much about the act of breathing makes it very hard to do...

I was completely stunned by this book when I read it. Firstly that I'd never heard of the syndrome or even imagined such a thing could exist and then once the horrors had sunk in, with it came all the emotion for those families who have to live with this sword of Damocles hanging over them. I found the Mad Cow section almost too terrifying to read.

Yes, Jem - I know what you mean - I can imagine this book could actually cause sleeplessness itself - and I find that about breathing too! I thought it was just me.

DGR: yes, it is utterly horrifying - and the thought of living with it in the family, and the way it comes over from the descriptions is just too awful...Not got to the Mad Cow section yet - I shall continue with trepidation.

Me too! It's got to be close to ten years since I read a newspaper article on a young man afflicted with prion disease (CJv). The picture of the boy reminded me so much of my son and the article was so heartbreaking that I swore off serving beef until there is adequate screening of cattle. I miss the beef, but any time I feel myself wavering I reread that now-yellowed-with-age newspaper clipping.

I do realize that the chances of a problem are extremely remote, Clare... I was just so traumatized by that news article. Poor wee boy. His name was Arnaud Eboli and I can see his face to this day - he looked so much like my son Jon.

Yes, I agree, Susan. It doesn't matter how remote the risk if you can identify with the victims.

A 15 year old was the first victim of vCJD in the UK and she lived very close to us in north Wales - which made it more real to us, somehow.

But Roger Tomkins, whose 24 year old daughter Clare died from the diease, maybe speaks for all the victims, and maybe for the rest of us too: "When I drive through London, I look at football matches and see hundreds and hundreds of thousands of people, and I think, 'Why my daughter?' As I am sure the other families think the same. It is such a minuscule risk, but it has happened. And the results of that risk are terrible."

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About Me

Clare DudmanClare Dudman is the author of four novels: Edge of Danger (Putnam), One Day the Ice Will Reveal Its Dead/Wegener's Jigsaw (Viking/Sceptre), 98 Reasons For Being ((Viking/Sceptre) and A Place of Meadows and Tall Trees (Seren) - and numerous short stories. Further details on this website.